Document Detail


Early bone marrow transplantation in an infant with Wiskott-Aldrich syndrome.
MedLine Citation:
PMID:  1793157     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The Wiskott-Aldrich Syndrome (WAS) is a rare X-linked immunohematological disorder characterized by eczema, profound thrombocytopenia, and progressive immunodeficiency. Severe hemorrhage, overwhelming sepsis, or lymphoreticular malignancy usually cause death in childhood. Recently, bone marrow transplantation (BMT) has been curative in some well-established cases, but there is no general agreement about the place of BMT in infants with WAS before the development of significant immunological abnormalities. We describe the successful use of early histocompatible BMT in a 10-month-old infant in whom WAS was diagnosed on the basis of eczema, thrombocytopenia, small platelets, and raised serum immunoglobulin A (Ig) and IgE, but before the development of immunodeficiency as evidenced clinically by recurrent infections, or immunologically by low serum IgM or consistently abnormal lymphocyte responses to mitogens. After an unstable period for several weeks posttransplantation when he developed marked hepatomegaly and severe interstitial pneumonitis, he made a good recovery. His eczema and thrombocytopenia resolved and he has shown no clinical or laboratory evidence of immunodeficiency. It is now over 2 years since his BMT. Because of the poor prognosis of WAS, where a histocompatible donor is available, BMT at the earliest opportunity, despite the inherent risks of such a procedure, may be the best option for an infant with WAS.
Authors:
L J Beard; I R Toogood; C C Pearson; A Ferrante
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The American journal of pediatric hematology/oncology     Volume:  13     ISSN:  0192-8562     ISO Abbreviation:  Am J Pediatr Hematol Oncol     Publication Date:  1991  
Date Detail:
Created Date:  1992-04-02     Completed Date:  1992-04-02     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7908071     Medline TA:  Am J Pediatr Hematol Oncol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  310-4     Citation Subset:  IM    
Affiliation:
Department of Immunology, Adelaide Children's Hospital, South Australia.
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MeSH Terms
Descriptor/Qualifier:
Age Factors
Bone Marrow Transplantation* / adverse effects
Follow-Up Studies
Humans
Immunocompromised Host
Immunoglobulins / analysis
Infant
Leukocyte Count
Male
Platelet Count
Pulmonary Fibrosis / etiology
Wiskott-Aldrich Syndrome / blood,  immunology,  surgery*
Chemical
Reg. No./Substance:
0/Immunoglobulins

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